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Malignant (lethal) midline granuloma (LMG)

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    Synonyms: Granuloma gangrenescens, malignant granuloma, midline malignant reticulosis granuloma, polymorphic reticulosi
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    Definition and clinical picture

    Very rare disorder with destruction of tissue structures localised in the midline region of the head such as the nose, palate, nasopharynx and larynx. More recent molecular-biological and immunological examination suggests it be considered as a variant of malignant T-cell type non-Hodgkin lymphoma; in the literature, designated as nTL-LMG (nasal T-cell lymphoma type lethal midline granuloma). In a large number of patients, identification of Epstein-Barr virus (EBV) genome in tumour tissue and serum. EBV aetiology

    Proof - Histopathological findings

    Serological proof of EBV infection: EBV-VCA-IgG and EBNA (nuclear antigen).

    Genotypical proof of re-arrangement of T-cell receptor genes and parts of the EBV genome in tumour tissue.

    Histologically: Diffuse proliferation of atypical lymphatic cells with irregular, hyperchromatic nuclei. Focal blast cells with clear mitotic activity, confluent tumor necroses in some areas. Tumour infiltration of vessels. The majority of tumour cells with immunohistochemical expression of T-lymphocyte antigen CD3. Immunohistochemical EBV diagnosis.

    Differential diagnosis

    Wegener's granulomatosis
    non-Hodgkin lymphoma of the nose and the paranasal sinuses (sinonasal NHL)

    Treatment and prognosis

    So far no defined treatment protocols. Immediate high-dose radiotherapy, and adjuvant chemotherapy, if necessary, are advised.

    Poor prognosis. Depending on the individual study and treatment protocols, the median survival rate is 6 months, the 5-year survival rate 22%.


    sources

    • Ullrich D, Wildfang I, Karstens JH, Schroder M (1999)   Nasales T-Zell-Lymphom- Typ letales Mittelliniengranulom   HNO. 1999 May;47(5):490-3